Figure: carnitine biosynthesis

Biochemistry

• carnitine biosynthesis 1
  • enzyme: methyltransferase
  • substrate: protein + 3 S-adenosylmethionine
  • product: protein with lys[CH3]3) + 3 S-adenosylhomocysteine
• carnitine biosynthesis 2
  • enzyme: protease
  • substrate: protein with lys[CH3]3 + H2O
  • product: peptide proteolyticfragment + trimethyllysine
• carnitine biosynthesis 3
  • enzyme: trimethyllysine hydroxylase
  • substrate: trimethyllysine + alpha-ketoglutarate + O2
  • product: beta-hydroxy trimethyllysine + succinate + CO2
• carnitine biosynthesis 4
  • enzyme: beta-hydroxy trimethyllysine aldolase
  • substrate: beta-hydroxy trimethyllysine
  • product: gamma-trimethylaminobutyraldehyde + glycine
• carnitine biosynthesis 5
  • enzyme: aldehyde dehydrogenase
  • substrate: gamma-trimethylaminobutyraldehyde + NAD + H2O
  • product: gamma-butyrobetaine + NADH
• carnitine biosynthesis 6
  • enzyme: gamma-butyrobetaine hydroxylase
  • substrate: gamma-butyrobetaine + alpha-ketoglutarate + O2
  • product: carnitine + succinate + CO2